Neonatal epidermolysis bullosa: a clinical practice guideline.

DEBRA International is undertaking a long-term initiative to develop clinical practice guidelines (CPGs) for epidermolysis bullosa (EB), to -improve the clinical care of people living with EB. Current neonatal care is based on evidence, clinical expertise and trial and error, with collaboration between the EB specialist team, parent or carer and patient, and is dependent on the neonate's individual presentation and type of EB. Early intervention based on research and clinical practice is needed to establish a foundation of knowledge to guide international practitioners to create and improve standards of care and to be able to work effectively with those newly diagnosed with EB.

Retrospective longitudinal study of osteoporosis in adults with recessive dystrophic epidermolysis bullosa.

This retrospective study looks at bone mineral density of people with recessive dystrophic epidermolysis bullosa as assessed by dual-energy x-ray absorptiometry. Data were collected in 34 patients aged 16-35 years. Statistical analysis showed immobility, low body mass index, and pubertal delay was associated with osteoporosis.

Quality of life among adults with epidermolysis bullosa living with a gastrostomy tube since childhood.

Epidermolysis bullosa (EB) is a rare genetic condition characterized by blistering to the skin and internal mucous membranes arising from mild mechanical trauma. The impact on those affected can be significant. They might have increased nutritional requirements because of blistering, chronic wounds, infection, and loss of exudates, and nutritional intake might be compromised because of oropharyngeal blistering and strictures, resulting in malnutrition in many patients.